Akila B. Ramaraj, Hannah V. Breitschopf, Kylie I. Holden, Vikas S. Gupta, Carrie L. Foster, Ashley H. Ebanks, Chase M. Miller, Terry L. Buchmiller, Matthew T. Harting, Rebecca A. Stark, CDH Study Group
Seattle Children’s Hospital and UConn Health. McGovern Medical School at the University of Texas Health Science Center and Children’s Memorial Hermann Hospital. Boston Children’s Hospital.
United States
Journal of Pediatrics
J Pediatr 2025;
DOI: 10.1016/j.jpeds.2025.114622
Abstract
Objectives: To evaluate long-term, post-discharge, ipsilateral lung perfusion patterns and to identify associated factors among patients with congenital diaphragmatic hernia (CDH).
Study design: A long-term follow-up database was created to assimile data across four centers, covering patients with left-sided CDH managed as outpatients between 2010 to 2021. Outcomes from discharge to age 5, focusing on ipsilateral lung perfusion at 2 and 5 years, were analyzed using both univariable and multivariable generalized linear modeling.
Results: Among the 258 patients with left-sided CDH, 213 (83%) and 100 (39%) had 2- and 5-year follow-up data, respectively. Of these, 173 patients (68%) had low-risk (A/B) defects. At discharge, 58 (22.5%) needed supplemental oxygen and 56 (21.7%) had pulmonary hypertension on echocardiogram. Perfusion data were available for 121 (47%) patients at 2 years and 54 (21%) at 5 years. Compared with the normal left lung perfusion index of 45-50% at 2 years, patients with CDH had abnormal ipsilateral perfusion deficits, with a median of 37.0% for the cohort, and a median ipsilateral perfusion of 39.0% for low-risk defects and 31.0% for high-risk defects, an absolute difference of 8.0 (P<0.001). In the longitudinal multivariable analysis, only ipsilateral perfusion at 2 years (p<0.001) and CDH defect stage (p=0.025) were significantly associated with ipsilateral perfusion at 5 years.
Conclusions: All patients with CDH had abnormal ipsilateral perfusion at 2 and 5 years, with defect size as the only factor associated with perfusion. Pulmonary hypertension was not associated with perfusion. Further clinical correlation is needed. These findings may help when counseling families on long-term outcomes.
Category
Class III. Pulmonary Hypertension Associated with Lung Hypoplasia
Diagnostic Testing for Pulmonary Vascular Disease. Non-invasive Testing
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
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